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Edwards建立了主动脉弓畸形模型，使对主动脉弓的5种类型容易理解（图9-22）。 | [
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【病理解剖】（一）主动脉双弓主动脉双弓畸形是由于任何部分的左弓或右弓未正常退化所致。 | [
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双侧第4鳃动脉弓均存在并形成主动脉弓。 | [
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升主动脉正常，在心包膜外分为左、右两支主动脉弓。 | [
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左侧主动脉弓在气管前方从右向左行走，越过左主支气管，在脊柱左侧与右侧主动脉弓汇合成降主动脉。 | [
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右侧主动脉弓跨越右侧主支气管在脊柱前方、食管后方，越过中线向左向下行，与左侧主动脉弓汇合成降主动脉。 | [
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双侧主动脉弓均存在并完全包绕气管和食管，每个弓分别发出两根大动脉。 | [
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绝大多数的动脉导管位于左侧。 | [
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在约90%的患儿，右弓较左弓粗大且位置较高。 | [
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有时左弓在颈总动脉和锁骨下动脉间或在左锁骨下动脉远端出现闭锁。 | [
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（二）右位主动脉弓伴左侧锁骨下动脉迷走右弓伴左锁骨下动脉迷走为胚胎发育时左弓在左颈总和左锁骨下之间退化所致，常不伴有其他心内畸形。 | [
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左锁骨下动脉起源于第Ⅳ腮弓，在主动脉弓后方发出，在食管后方走行并供应左上肢。 | [
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左侧动脉导管在左锁骨下动脉起始部的主动脉憩室发出，连接与左肺动脉，将主动脉及憩室拉向前方形成血管环并压迫食管和气管。 | [
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通常该血管环较松，但当动脉导管较短时可引起压迫症状。 | [
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【临床表现】表现为气管压迫，可有喘鸣、气急和犬吠样咳嗽并在运动和进食时加剧。 | [
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食管压迫症状较少见，在婴儿可有呕吐、噫塞和喂养困难，在儿童可表现为吞咽困难。 | [
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多数在儿童期随着主动脉及锁骨上动脉的增大而出现症状。 | [
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在右位主动脉弓伴左侧锁骨下动脉迷走时，由于动脉导管较短或主动脉憩室较大时可出现同样的气管、食管压迫症状。 | [
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图9-22Edwards主动脉弓畸形模型DA：降主动脉AA：升主动脉RS：右锁骨下动脉LS：左锁骨下动脉RC：右颈总动脉LC：左颈总动脉E：为食管T：为气管A.主动脉双弓；B.正常左位主动脉弓；C.左弓右锁骨下动脉迷走；D.右位主动脉弓；E.右弓左锁骨下动脉迷走听诊可及气道的喘鸣音，可有轻度的青紫出现，喜欢背屈、仰颈体位。 | [
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【辅助检查】（一）胸部X线检查右位主动脉弓为血管环诊断的重要提示，正常情况下气管稍偏右侧，在平片无法清晰显示主动脉弓时，如平片上气管居中提示右位主动脉弓的存在。 | [
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双主动脉弓病例可显示双侧主动脉弓球形隆起，右侧更为明显。 | [
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食管造影检查可显示在胸椎第3、4水平上段食管两侧压迹，右主动脉弓造成的压迹较大且位置较高，左主动脉弓造成的压迹较小且位置较低。 | [
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CT检查可能显示气管腔受压迫的征象。 | [
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左锁骨下动脉迷走患儿食管造影可显示食管后壁受血管压迫呈现斜行或螺旋形的压迹。 | [
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婴儿作食管造影检查宜用碘油或水溶性造影剂，因钡剂造影剂如吸入气管支气管内，有加重呼吸困难或引致吸入性肺炎的危险。 | [
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"start_offset": 40,
"end_offset": 44,
"label": "sym"
},
{
"id": 6,
"entity": "吸入性肺炎",
"start_offset": 47,
"end_offset": 52,
"label": "dis"
}
] |
（二）支气管镜检查支气管镜检查可以明确气管受压迫的部位，并可在受压处观察到血管搏动，但引起的呼吸道黏膜创伤和水肿可加重呼吸道梗阻，必须十分慎重。 | [
{
"id": 0,
"entity": "支气管镜检查",
"start_offset": 3,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "支气管镜检查",
"start_offset": 9,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "气管",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "血管",
"start_offset": 37,
"end_offset": 39,
"label": "bod"
},
{
"id": 4,
"entity": "呼吸道黏膜创伤",
"start_offset": 46,
"end_offset": 53,
"label": "dis"
},
{
"id": 5,
"entity": "水肿",
"start_offset": 54,
"end_offset": 56,
"label": "dis"
},
{
"id": 6,
"entity": "呼吸道梗阻",
"start_offset": 59,
"end_offset": 64,
"label": "dis"
}
] |
（三）主动脉造影检查主动脉造影是确诊主动脉弓及其分支畸形最升主动脉诊断方法。 | [
{
"id": 0,
"entity": "主动脉造影检查",
"start_offset": 3,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "主动脉造影",
"start_offset": 10,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "主动脉弓",
"start_offset": 18,
"end_offset": 22,
"label": "dis"
},
{
"id": 3,
"entity": "升主动脉",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
}
] |
于升主动脉内插入导管，注入造影剂进行主动脉及其分支造影，双向电影摄片检查可显示主动脉弓及其分支的起源、走向、粗细和其他异常，从而明确诊断。 | [
{
"id": 0,
"entity": "导管",
"start_offset": 8,
"end_offset": 10,
"label": "equ"
},
{
"id": 1,
"entity": "造影剂",
"start_offset": 13,
"end_offset": 16,
"label": "dru"
},
{
"id": 2,
"entity": "主动脉及其分支造影",
"start_offset": 18,
"end_offset": 27,
"label": "pro"
},
{
"id": 3,
"entity": "双向电影摄片检查",
"start_offset": 28,
"end_offset": 36,
"label": "pro"
},
{
"id": 4,
"entity": "主动脉弓",
"start_offset": 39,
"end_offset": 43,
"label": "bod"
}
] |
【治疗】对所有有症状的血管环患儿均应进行外科手术治疗。 | [
{
"id": 0,
"entity": "血管环",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "外科手术",
"start_offset": 20,
"end_offset": 24,
"label": "pro"
}
] |
手术治疗时需经左胸径路，双弓畸形者应将较小的主动脉弓离断（通常为左侧），右位主动脉弓伴左侧锁骨下动脉迷走者则离断动脉导管韧带以松开气管、食管的压迫。 | [
{
"id": 0,
"entity": "左胸径",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "双弓畸形",
"start_offset": 12,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "主动脉弓",
"start_offset": 22,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "右位主动脉弓",
"start_offset": 36,
"end_offset": 42,
"label": "dis"
},
{
"id": 4,
"entity": "左侧锁骨下动脉迷走",
"start_offset": 43,
"end_offset": 52,
"label": "dis"
},
{
"id": 5,
"entity": "动脉导管韧带",
"start_offset": 56,
"end_offset": 62,
"label": "bod"
},
{
"id": 6,
"entity": "气管",
"start_offset": 65,
"end_offset": 67,
"label": "bod"
},
{
"id": 7,
"entity": "食管",
"start_offset": 68,
"end_offset": 70,
"label": "bod"
}
] |
手术患者在术后仍有肺功能异常，可能与压迫后气道器质性残余梗阻有关。 | [
{
"id": 0,
"entity": "肺",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "气道器质性残余梗阻",
"start_offset": 21,
"end_offset": 30,
"label": "dis"
}
] |
七、淋巴管肉瘤淋巴管肉瘤（lymphangiosarcoma）在小儿极为罕见，高度恶性。 | [
{
"id": 0,
"entity": "淋巴管肉瘤",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "淋巴管肉瘤",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "lymphangiosarcoma",
"start_offset": 13,
"end_offset": 30,
"label": "dis"
}
] |
肿瘤起源于淋巴管内皮，其原因与放疗等原因引起的慢性淋巴水肿有关，先天型淋巴水肿也可发生。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "淋巴管内皮",
"start_offset": 5,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "放疗",
"start_offset": 15,
"end_offset": 17,
"label": "pro"
},
{
"id": 3,
"entity": "慢性淋巴水肿",
"start_offset": 23,
"end_offset": 29,
"label": "dis"
},
{
"id": 4,
"entity": "先天型淋巴水肿",
"start_offset": 32,
"end_offset": 39,
"label": "dis"
}
] |
肿瘤内见不规则的脉管结构，衬以恶性内皮细胞，细胞呈卵圆形及棱形胞质。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "脉管",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "肿瘤内见不规则的脉管结构",
"start_offset": 0,
"end_offset": 12,
"label": "sym"
},
{
"id": 3,
"entity": "恶性内皮细胞",
"start_offset": 15,
"end_offset": 21,
"label": "bod"
},
{
"id": 4,
"entity": "衬以恶性内皮细胞",
"start_offset": 13,
"end_offset": 21,
"label": "sym"
},
{
"id": 5,
"entity": "细胞",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
},
{
"id": 6,
"entity": "细胞呈卵圆形及棱形胞质",
"start_offset": 22,
"end_offset": 33,
"label": "sym"
}
] |
第Ⅷ因子相关抗原也是本瘤的一个标志。 | [
{
"id": 0,
"entity": "抗原",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
}
] |
肿瘤呈现一个坚韧的肿块，如色青的瘀斑，高出皮面相近的皮肤、皮下组织有纤维化肿瘤多发生于四肢，向近远端发展。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "肿块",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "色青的瘀斑",
"start_offset": 13,
"end_offset": 18,
"label": "sym"
},
{
"id": 3,
"entity": "皮面",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 4,
"entity": "高出皮面",
"start_offset": 19,
"end_offset": 23,
"label": "sym"
},
{
"id": 5,
"entity": "皮肤",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 6,
"entity": "皮下组织",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
},
{
"id": 7,
"entity": "相近的皮肤、皮下组织有纤维化",
"start_offset": 23,
"end_offset": 37,
"label": "sym"
},
{
"id": 8,
"entity": "肿瘤",
"start_offset": 37,
"end_offset": 39,
"label": "dis"
},
{
"id": 9,
"entity": "四肢",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
}
] |
如有肿瘤症状，即作根治手术。 | [
{
"id": 0,
"entity": "肿瘤",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "手术",
"start_offset": 11,
"end_offset": 13,
"label": "pro"
}
] |
四、并发症儿科纤维支气管镜检查的并发症较少见，大多因麻醉不当或操作不熟练所致。 | [
{
"id": 0,
"entity": "儿科",
"start_offset": 5,
"end_offset": 7,
"label": "dep"
},
{
"id": 1,
"entity": "纤维支气管镜检查",
"start_offset": 7,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "麻醉",
"start_offset": 26,
"end_offset": 28,
"label": "pro"
}
] |
（一）喉水肿是最常见的并发症，多在术后2小时内出现。 | [
{
"id": 0,
"entity": "喉水肿",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
}
] |
应选择粗细合适的纤维支气管镜，操作动作轻柔，检查时间不宜过长，术毕要观察半小时左右再送出手术室。 | [
{
"id": 0,
"entity": "纤维支气管镜",
"start_offset": 8,
"end_offset": 14,
"label": "equ"
},
{
"id": 1,
"entity": "手术室",
"start_offset": 44,
"end_offset": 47,
"label": "dep"
}
] |
一旦出现喉水肿，在氧气吸入的同时，静脉注射地塞米松、雾化吸入肾上腺素和布地奈德能有效防止喉梗阻的发生。 | [
{
"id": 0,
"entity": "喉水肿",
"start_offset": 4,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "氧气吸入",
"start_offset": 9,
"end_offset": 13,
"label": "pro"
},
{
"id": 2,
"entity": "静脉注射",
"start_offset": 17,
"end_offset": 21,
"label": "pro"
},
{
"id": 3,
"entity": "地塞米松",
"start_offset": 21,
"end_offset": 25,
"label": "dru"
},
{
"id": 4,
"entity": "雾化吸入",
"start_offset": 26,
"end_offset": 30,
"label": "pro"
},
{
"id": 5,
"entity": "肾上腺素",
"start_offset": 30,
"end_offset": 34,
"label": "dru"
},
{
"id": 6,
"entity": "布地奈德",
"start_offset": 35,
"end_offset": 39,
"label": "dru"
},
{
"id": 7,
"entity": "喉梗阻",
"start_offset": 44,
"end_offset": 47,
"label": "sym"
}
] |
（二）喉痉挛多由于麻醉不充分，刺激喉部发生。 | [
{
"id": 0,
"entity": "喉痉挛",
"start_offset": 3,
"end_offset": 6,
"label": "sym"
},
{
"id": 1,
"entity": "麻醉",
"start_offset": 9,
"end_offset": 11,
"label": "pro"
},
{
"id": 2,
"entity": "喉部",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
}
] |
加深麻醉或对喉头进行表面麻醉后可消失。 | [
{
"id": 0,
"entity": "麻醉",
"start_offset": 2,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "喉头",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "表面麻醉",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
}
] |
（三）黏膜出血多由于气道黏膜炎症、负压吸引及取异物、活检创伤所致。 | [
{
"id": 0,
"entity": "黏膜出血",
"start_offset": 3,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "气道黏膜炎症",
"start_offset": 10,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "负压吸引",
"start_offset": 17,
"end_offset": 21,
"label": "pro"
},
{
"id": 3,
"entity": "活检",
"start_offset": 26,
"end_offset": 28,
"label": "pro"
}
] |
用纤维支气管镜直接压迫出血处或注入少量1∶10000肾上腺素液多能止血。 | [
{
"id": 0,
"entity": "纤维支气管镜",
"start_offset": 1,
"end_offset": 7,
"label": "equ"
},
{
"id": 1,
"entity": "压迫出血处",
"start_offset": 9,
"end_offset": 14,
"label": "pro"
},
{
"id": 2,
"entity": "肾上腺素液",
"start_offset": 26,
"end_offset": 31,
"label": "dru"
}
] |
少数患儿可引起大咯血，甚至气道堵塞、窒息死亡。 | [
{
"id": 0,
"entity": "大咯血",
"start_offset": 7,
"end_offset": 10,
"label": "sym"
},
{
"id": 1,
"entity": "气道堵塞",
"start_offset": 13,
"end_offset": 17,
"label": "sym"
},
{
"id": 2,
"entity": "窒息死亡",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
}
] |
活检时尤其应该小心谨慎，一旦发生严重出血，应及时抽吸积血，并肌内注射或经镜管内滴入垂体后叶素、酚磺乙胺等药物。 | [
{
"id": 0,
"entity": "活检",
"start_offset": 0,
"end_offset": 2,
"label": "pro"
},
{
"id": 1,
"entity": "严重出血",
"start_offset": 16,
"end_offset": 20,
"label": "sym"
},
{
"id": 2,
"entity": "抽吸积血",
"start_offset": 24,
"end_offset": 28,
"label": "pro"
},
{
"id": 3,
"entity": "肌内注射",
"start_offset": 30,
"end_offset": 34,
"label": "pro"
},
{
"id": 4,
"entity": "镜管",
"start_offset": 36,
"end_offset": 38,
"label": "equ"
},
{
"id": 5,
"entity": "垂体后叶素",
"start_offset": 41,
"end_offset": 46,
"label": "dru"
},
{
"id": 6,
"entity": "酚磺乙胺",
"start_offset": 47,
"end_offset": 51,
"label": "dru"
}
] |
（四）缺氧或发绀纤维支气管镜术中可引起短暂性PaO<sub>2</sub>下降，如频繁吸引可造成通气不足而缺氧，其他原因包括原有肺功能不全、呼吸抑制、支气管痉挛、用药过量等。 | [
{
"id": 0,
"entity": "缺氧",
"start_offset": 3,
"end_offset": 5,
"label": "sym"
},
{
"id": 1,
"entity": "发绀",
"start_offset": 6,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "纤维支气管镜术",
"start_offset": 8,
"end_offset": 15,
"label": "pro"
},
{
"id": 3,
"entity": "PaO<sub>2</sub>",
"start_offset": 22,
"end_offset": 37,
"label": "ite"
},
{
"id": 4,
"entity": "通气不足而缺氧",
"start_offset": 48,
"end_offset": 55,
"label": "sym"
},
{
"id": 5,
"entity": "原有肺功能不全",
"start_offset": 62,
"end_offset": 69,
"label": "dis"
},
{
"id": 6,
"entity": "呼吸抑制",
"start_offset": 70,
"end_offset": 74,
"label": "dis"
},
{
"id": 7,
"entity": "支气管痉挛",
"start_offset": 75,
"end_offset": 80,
"label": "dis"
}
] |
可经纤维支气管镜活检孔给氧或口鼻腔给氧，新生儿超细纤维支气管镜检查时可通过附加管道气管内给氧。 | [
{
"id": 0,
"entity": "纤维支气管镜活检孔",
"start_offset": 2,
"end_offset": 11,
"label": "equ"
},
{
"id": 1,
"entity": "给氧",
"start_offset": 11,
"end_offset": 13,
"label": "pro"
},
{
"id": 2,
"entity": "口鼻腔",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 3,
"entity": "给氧",
"start_offset": 17,
"end_offset": 19,
"label": "pro"
},
{
"id": 4,
"entity": "新生儿超细纤维支气管镜检查",
"start_offset": 20,
"end_offset": 33,
"label": "pro"
},
{
"id": 5,
"entity": "附加管道",
"start_offset": 37,
"end_offset": 41,
"label": "equ"
},
{
"id": 6,
"entity": "气管",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 7,
"entity": "给氧",
"start_offset": 44,
"end_offset": 46,
"label": "pro"
}
] |
（五）其他如气胸或纵隔气肿、麻醉药物过敏、继发感染、心动过速等。 | [
{
"id": 0,
"entity": "气胸",
"start_offset": 6,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "纵隔气肿",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "麻醉药物过敏",
"start_offset": 14,
"end_offset": 20,
"label": "dis"
},
{
"id": 3,
"entity": "继发感染",
"start_offset": 21,
"end_offset": 25,
"label": "dis"
},
{
"id": 4,
"entity": "心动过速",
"start_offset": 26,
"end_offset": 30,
"label": "dis"
}
] |
纤维支气管镜的应用价值和前景毋容置疑，具体应用中的主要问题是确保安全。 | [
{
"id": 0,
"entity": "纤维支气管镜",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
}
] |
三、新生儿贫血的治疗应根据新生儿贫血的不同类型、严重程度，给予相应治疗。 | [
{
"id": 0,
"entity": "新生儿贫血",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "新生儿贫血",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
}
] |
对无症状的轻、中度贫血，以病因治疗为主，并补充维生素E和铁剂等，尽量减少输血。 | [
{
"id": 0,
"entity": "贫血",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "维生素E",
"start_offset": 23,
"end_offset": 27,
"label": "dru"
},
{
"id": 2,
"entity": "铁剂",
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"label": "dru"
},
{
"id": 3,
"entity": "输血",
"start_offset": 36,
"end_offset": 38,
"label": "pro"
}
] |
对重度贫血、急性失血性贫血，应进行输血治疗。 | [
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"id": 0,
"entity": "重度贫血",
"start_offset": 1,
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{
"id": 1,
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] |
（一）失血性贫血的治疗1.纠正低血容量性休克急性失血可导致休克，须紧急处理，应先给生理盐水扩容，20ml/kg，快速静脉滴注。 | [
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"id": 0,
"entity": "失血性贫血",
"start_offset": 3,
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"label": "dis"
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{
"id": 1,
"entity": "低血容量性休克",
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"label": "dis"
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{
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"entity": "急性失血",
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{
"id": 3,
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"label": "sym"
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"entity": "生理盐水",
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"label": "dru"
},
{
"id": 5,
"entity": "静脉滴注",
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] |
如血压仍不稳定，可适当增加扩容量。 | [
{
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"entity": "血压",
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] |
如血压稳定，给维持量补液。 | [
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] |
2.纠正贫血经过扩容，血压稳定后，如贫血较明显者，考虑输血，以纠正贫血。 | [
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"entity": "贫血",
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"label": "dis"
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{
"id": 1,
"entity": "血压",
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{
"id": 4,
"entity": "贫血",
"start_offset": 33,
"end_offset": 35,
"label": "dis"
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] |
（二）溶血性贫血的治疗详见本篇第十六节新生儿溶血病。 | [
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"entity": "溶血性贫血",
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] |
（三）早产儿贫血的治疗1.使用重组人促红细胞生成素（rhEPO）（1）预防：对出生体重小于1500g的早产儿，生后7天，给rhEPO200U/kg，每周3次，皮下注射或静脉滴注，疗程4周。 | [
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"entity": "早产儿贫血",
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"end_offset": 8,
"label": "dis"
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{
"id": 1,
"entity": "重组人促红细胞生成素",
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{
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{
"id": 3,
"entity": "皮下注射",
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"label": "pro"
},
{
"id": 4,
"entity": "静脉滴注",
"start_offset": 84,
"end_offset": 88,
"label": "pro"
}
] |
（2）治疗：对已发生贫血者，给rhEPO300U/kg，每周3次，疗程4周。 | [
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"entity": "贫血",
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"label": "dis"
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{
"id": 1,
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使用rhEPO可缓解贫血严重程度，减少输血次数，但不能避免输血。 | [
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"id": 0,
"entity": "rhEPO",
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{
"id": 1,
"entity": "贫血",
"start_offset": 10,
"end_offset": 12,
"label": "dis"
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] |
2.补充维生素E和铁剂在使用rhEPO的同时给维生素E10mg/d，分2次口服，1周后给3%硫酸亚铁3mg/（kg•d），分2次口服，每周增加2mg/（kg•d），至7mg/（kg•d）维持。 | [
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"id": 0,
"entity": "维生素E",
"start_offset": 4,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
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"start_offset": 9,
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"label": "dru"
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{
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"entity": "rhEPO",
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"id": 3,
"entity": "维生素E",
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"label": "dru"
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{
"id": 4,
"entity": "3%硫酸亚铁",
"start_offset": 44,
"end_offset": 50,
"label": "dru"
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] |
3.输血如血红蛋白低于80g/L并出现以下情况者需输血：胎龄小于30周，安静时呼吸增快＞50次/分、心率加快＞160次/分，进食易疲劳，每天体重增加＜25g，血乳酸＞1.8mmol/L。 | [
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"id": 0,
"entity": "血红蛋白",
"start_offset": 5,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "输血",
"start_offset": 25,
"end_offset": 27,
"label": "pro"
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{
"id": 2,
"entity": "呼吸",
"start_offset": 39,
"end_offset": 41,
"label": "ite"
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{
"id": 3,
"entity": "心率",
"start_offset": 50,
"end_offset": 52,
"label": "ite"
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{
"id": 4,
"entity": "体重",
"start_offset": 70,
"end_offset": 72,
"label": "ite"
},
{
"id": 5,
"entity": "血乳酸",
"start_offset": 79,
"end_offset": 82,
"label": "ite"
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] |
输血量每次10～15ml/kg。 | [
{
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"entity": "输血量",
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"label": "ite"
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] |
（四）输血疗法1.输血量根据贫血类型及轻重缓急而不同，也可根据公式计算，所需全血量（ml）=体重（kg）×（预期达到Hb-实际Hb）×6（6ml血提高1gHb），如输压缩红细胞，为全血量的1/2。 | [
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"id": 0,
"entity": "贫血",
"start_offset": 14,
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"label": "dis"
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] |
2.注意事项重度贫血输血速度应缓慢。 | [
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"entity": "重度贫血",
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{
"id": 1,
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"start_offset": 10,
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] |
充血性心力衰竭患儿，为防止输血引起血容量进一步增加，输血前可静脉注射呋塞米0.5mg/kg。 | [
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"id": 0,
"entity": "充血性心力衰竭",
"start_offset": 0,
"end_offset": 7,
"label": "dis"
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{
"id": 1,
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{
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{
"id": 3,
"entity": "输血",
"start_offset": 26,
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{
"id": 4,
"entity": "静脉注射",
"start_offset": 30,
"end_offset": 34,
"label": "pro"
},
{
"id": 5,
"entity": "呋塞米",
"start_offset": 34,
"end_offset": 37,
"label": "dru"
}
] |
（五）其他治疗要积极治疗原发病及并发症。 | [
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"id": 0,
"entity": "原发病",
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{
"id": 1,
"entity": "并发症",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
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] |
二、GINA治疗方案（一）GINA治疗方案的形成与演变1994年在美国国立卫生院心肺血液研究所与世界卫生组织的共同努力下，17个国家的30多位专家组成小组，制定了关于哮喘管理和预防的全球策略，即《全球哮喘防治创议》（GlobalInitiativeforAsthma，GINA），用来规范哮喘的防治。 | [
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"id": 0,
"entity": "GINA治疗",
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{
"id": 1,
"entity": "GINA治疗",
"start_offset": 13,
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{
"id": 2,
"entity": "心",
"start_offset": 40,
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{
"id": 3,
"entity": "肺",
"start_offset": 41,
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{
"id": 4,
"entity": "血液",
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{
"id": 5,
"entity": "哮喘",
"start_offset": 83,
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{
"id": 6,
"entity": "哮喘",
"start_offset": 144,
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"label": "dis"
}
] |
早期GINA是根据症状、气流受限的程度以及肺功能的改变，对哮喘病情进行严重程度的分级（即间歇、轻度持续、中度持续、重度持续），并根据分级采用相应的治疗方案。 | [
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{
"id": 2,
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但随着在临床上广泛的推广，也感觉哮喘严重程度既涉及疾病本身的严重性，也涉及其对治疗的反应。 | [
{
"id": 0,
"entity": "哮喘",
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] |
而且哮喘严重程度在具体某一哮喘患儿也不是一成不变的，可能在不同季节或环境改变后发生改变。 | [
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"entity": "哮喘",
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{
"id": 1,
"entity": "哮喘",
"start_offset": 13,
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"label": "dis"
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] |
所以，就哮喘管理的持续性而言，根据控制水平对哮喘进行分类更符合实际情况（表8-8）。 | [
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"id": 0,
"entity": "哮喘",
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"id": 1,
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表8-22喷射性雾化吸入用药注：普米克令舒可与0.9%生理盐水、特布他林、沙丁胺醇、色甘酸钠和溴化异丙托品混合使用表8-23儿童哮喘严重度分级注：①评估过去2～4周日间症状、夜间症状/憋醒、应急缓解药使用和活动受限情况；②患儿只要具有某级严重程度的任一项特点，就将其列为该级别；③任何级别严重程度，包括间歇状态，都可以出现严重的急性发作表8-24儿童哮喘控制水平分级注：①评估过去2～4周日间症状、夜间症状/憋醒、应急缓解药使用和活动受限情况；②出现任何一次急性发作都应复核维持治疗方案是否需要调整（二）确定长期治疗方案根据年龄分为5岁及以上儿童哮喘和5岁以下儿童哮喘的长期治疗方案。 | [
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"entity": "喷射性雾化",
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{
"id": 1,
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"end_offset": 21,
"label": "dru"
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{
"id": 2,
"entity": "生理盐水",
"start_offset": 27,
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"label": "dru"
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{
"id": 3,
"entity": "特布他林",
"start_offset": 32,
"end_offset": 36,
"label": "dru"
},
{
"id": 4,
"entity": "沙丁胺醇",
"start_offset": 37,
"end_offset": 41,
"label": "dru"
},
{
"id": 5,
"entity": "色甘酸钠",
"start_offset": 42,
"end_offset": 46,
"label": "dru"
},
{
"id": 6,
"entity": "溴化异丙托品",
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"end_offset": 53,
"label": "dru"
},
{
"id": 7,
"entity": "儿童哮喘",
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"end_offset": 66,
"label": "dis"
},
{
"id": 8,
"entity": "儿童哮喘",
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"end_offset": 177,
"label": "dis"
},
{
"id": 9,
"entity": "儿童哮喘",
"start_offset": 280,
"end_offset": 284,
"label": "dis"
}
] |
如哮喘控制，并维持至少3个月，治疗方案可考虑降级，直至确定维持哮喘控制的最小剂量。 | [
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"entity": "哮喘",
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{
"id": 1,
"entity": "哮喘",
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"label": "dis"
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] |
（三）常用哮喘维持治疗药物吸入糖皮质激素（ICS）种类ICS治疗哮喘的高效性和局部选择性的主要化学基础是在于激素甾体核的16α和17α或17β位置上有一个亲脂基团的置换。 | [
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{
"id": 1,
"entity": "糖皮质激素",
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"label": "dru"
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{
"id": 2,
"entity": "ICS",
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{
"id": 3,
"entity": "ICS",
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},
{
"id": 4,
"entity": "哮喘",
"start_offset": 32,
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"label": "dis"
}
] |
当甾体核的D环上用亲脂基团替代可得到三种重要特性：①与激素受体有非常高度的亲和性，这是在呼吸道黏膜发挥作用所必需的；②能增加局部摄取（浓度）和延长在组织中储存时间；③全身吸收后，易被肝脏转化而快速灭活。 | [
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{
"id": 1,
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"label": "bod"
},
{
"id": 2,
"entity": "肝脏",
"start_offset": 91,
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"label": "bod"
}
] |
但一定程度的水溶性也十分重要，ICS必须首先溶解在气道黏液中，然后才能作用于气道组织，因而一个理想的ICS除了较强的脂溶性外，还需要一定的水溶性。 | [
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{
"id": 1,
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{
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{
"id": 3,
"entity": "ICS",
"start_offset": 50,
"end_offset": 53,
"label": "dru"
}
] |
ICS的局部/全身作用的比例取决于：①药物在气道中的局部活性；②下呼吸道与口咽部药物沉积之比；③药物经肺或胃肠道吸收和首过代谢的周身活性。 | [
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{
"id": 1,
"entity": "下呼吸道",
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{
"id": 2,
"entity": "口咽部",
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{
"id": 3,
"entity": "肺",
"start_offset": 51,
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},
{
"id": 4,
"entity": "胃肠道",
"start_offset": 53,
"end_offset": 56,
"label": "bod"
}
] |
目前临床上常用的ICS有以下三大类：（1）二丙酸倍氯米松（beclomethasonide，BDP）：如必可酮、贝可乐；BDP是丙酸倍氯米松（BMP）的前体，BMP比BDP具有更高的受体亲和力，BDP水溶性低，在肺组织中转化成BMP。 | [
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"entity": "ICS",
"start_offset": 8,
"end_offset": 11,
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{
"id": 1,
"entity": "二丙酸倍氯米松",
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{
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{
"id": 3,
"entity": "BDP",
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{
"id": 4,
"entity": "必可酮",
"start_offset": 52,
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"label": "dru"
},
{
"id": 5,
"entity": "贝可乐",
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{
"id": 6,
"entity": "BDP",
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},
{
"id": 7,
"entity": "丙酸倍氯米松",
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"end_offset": 70,
"label": "dru"
},
{
"id": 8,
"entity": "BMP",
"start_offset": 71,
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"label": "dru"
},
{
"id": 9,
"entity": "BMP",
"start_offset": 79,
"end_offset": 82,
"label": "dru"
},
{
"id": 10,
"entity": "BDP",
"start_offset": 83,
"end_offset": 86,
"label": "dru"
},
{
"id": 11,
"entity": "BDP",
"start_offset": 97,
"end_offset": 100,
"label": "dru"
},
{
"id": 12,
"entity": "肺组织",
"start_offset": 106,
"end_offset": 109,
"label": "bod"
},
{
"id": 13,
"entity": "BMP",
"start_offset": 113,
"end_offset": 116,
"label": "dru"
}
] |
肝脏灭活速度慢，并且在肝脏代谢后会产生另一种活性产物（倍氯米松）；因而全身不良反应相对较大。 | [
{
"id": 0,
"entity": "肝脏",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "肝脏",
"start_offset": 11,
"end_offset": 13,
"label": "bod"
}
] |
（2）布地奈德（budesonide，BUD）：普米克都保或pMDI、英福美；BUD比BDP有较高的受体亲和性和水溶性，而与BMP接近。 | [
{
"id": 0,
"entity": "布地奈德",
"start_offset": 3,
"end_offset": 7,
"label": "dru"
},
{
"id": 1,
"entity": "budesonide",
"start_offset": 8,
"end_offset": 18,
"label": "dru"
},
{
"id": 2,
"entity": "BUD",
"start_offset": 19,
"end_offset": 22,
"label": "dru"
},
{
"id": 3,
"entity": "普米克都保",
"start_offset": 24,
"end_offset": 29,
"label": "dru"
},
{
"id": 4,
"entity": "pMDI",
"start_offset": 30,
"end_offset": 34,
"label": "dru"
},
{
"id": 5,
"entity": "英福美",
"start_offset": 35,
"end_offset": 38,
"label": "dru"
},
{
"id": 6,
"entity": "BUD",
"start_offset": 39,
"end_offset": 42,
"label": "dru"
},
{
"id": 7,
"entity": "BDP",
"start_offset": 43,
"end_offset": 46,
"label": "dru"
},
{
"id": 8,
"entity": "BMP",
"start_offset": 62,
"end_offset": 65,
"label": "dru"
}
] |
BUD肝脏灭活速度较BMP快，肝脏通过两种代谢途径进行代谢，首过代谢为90%，半衰期2.8小时。 | [
{
"id": 0,
"entity": "BUD",
"start_offset": 0,
"end_offset": 3,
"label": "dru"
},
{
"id": 1,
"entity": "肝脏",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 2,
"entity": "BMP",
"start_offset": 10,
"end_offset": 13,
"label": "dru"
},
{
"id": 3,
"entity": "肝脏",
"start_offset": 15,
"end_offset": 17,
"label": "bod"
}
] |
图8-16≥5岁儿童哮喘的长期治疗方案（3）氟地卡松（fluticasonepropionate，FP）：如辅舒酮pMDI。 | [
{
"id": 0,
"entity": "儿童哮喘",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "氟地卡松",
"start_offset": 22,
"end_offset": 26,
"label": "dru"
},
{
"id": 2,
"entity": "fluticasonepropionate",
"start_offset": 27,
"end_offset": 48,
"label": "dru"
},
{
"id": 3,
"entity": "FP",
"start_offset": 49,
"end_offset": 51,
"label": "dru"
},
{
"id": 4,
"entity": "辅舒酮pMDI",
"start_offset": 54,
"end_offset": 61,
"label": "dru"
}
] |
FP与BDP一样水溶性低，但受体亲和力高；FP只通过一种代谢途径，首过代谢为99%，半衰期8～14小时。 | [
{
"id": 0,
"entity": "FP",
"start_offset": 0,
"end_offset": 2,
"label": "dru"
},
{
"id": 1,
"entity": "BDP",
"start_offset": 3,
"end_offset": 6,
"label": "dru"
},
{
"id": 2,
"entity": "FP",
"start_offset": 21,
"end_offset": 23,
"label": "dru"
}
] |
长半衰期增加了反复用药的危险性，可导致组织内药物高浓度；FP的长半衰期可能与其高亲脂性有关，可增加组织结合和分布容积。 | [
{
"id": 0,
"entity": "FP",
"start_offset": 28,
"end_offset": 30,
"label": "dru"
}
] |
应用ICS应注意根据年龄选择合适的吸入装置，以增加吸入效率（表8-8）。 | [
{
"id": 0,
"entity": "ICS",
"start_offset": 2,
"end_offset": 5,
"label": "dru"
}
] |
图8-17＜5岁儿童哮喘的长期治疗方案表8-25不同年龄的吸入策略（四）白三烯受体拮抗剂白三烯是人体三种必需脂肪酸之一的花生四烯酸的脂氧化酶代谢产物，包括LTA4</sub>、LTB4</sub>、LTC4</sub>、LTD4</sub>和LTE4</sub>；其中LTC4</sub>、LTD4</sub>和LTE4</sub>被称为“半胱氨酰白三烯”，因为它们都包含一个硫醚连接的肽，主要由嗜酸性粒细胞、肥大细胞、巨噬细胞、单核细胞和嗜碱粒细胞产生。 | [
{
"id": 0,
"entity": "儿童哮喘",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "白三烯受体拮抗剂",
"start_offset": 36,
"end_offset": 44,
"label": "dru"
},
{
"id": 2,
"entity": "花生四烯酸",
"start_offset": 60,
"end_offset": 65,
"label": "bod"
},
{
"id": 3,
"entity": "脂氧化酶",
"start_offset": 66,
"end_offset": 70,
"label": "mic"
},
{
"id": 4,
"entity": "嗜酸性粒细胞",
"start_offset": 198,
"end_offset": 204,
"label": "bod"
},
{
"id": 5,
"entity": "肥大细胞",
"start_offset": 205,
"end_offset": 209,
"label": "bod"
},
{
"id": 6,
"entity": "巨噬细胞",
"start_offset": 210,
"end_offset": 214,
"label": "bod"
},
{
"id": 7,
"entity": "单核细胞",
"start_offset": 215,
"end_offset": 219,
"label": "bod"
},
{
"id": 8,
"entity": "嗜碱粒细胞",
"start_offset": 220,
"end_offset": 225,
"label": "bod"
}
] |
半胱氨酰白三烯是引起哮喘慢性气道炎症的重要炎性介质之一。 | [
{
"id": 0,
"entity": "哮喘慢性气道炎症",
"start_offset": 10,
"end_offset": 18,
"label": "dis"
}
] |
孟鲁司特钠和扎鲁司特是口服的选择性白三烯受体拮抗剂，能特异性抑制半胱氨酰白三烯（CysLT1）受体，以阻断白三烯引起的气道炎症；与糖皮质激素合用，可减少激素用量。 | [
{
"id": 0,
"entity": "孟鲁司特钠",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "扎鲁司特",
"start_offset": 6,
"end_offset": 10,
"label": "dru"
},
{
"id": 2,
"entity": "选择性白三烯受体拮抗剂",
"start_offset": 14,
"end_offset": 25,
"label": "dru"
},
{
"id": 3,
"entity": "半胱氨酰白三烯（CysLT1）受体",
"start_offset": 32,
"end_offset": 49,
"label": "mic"
},
{
"id": 4,
"entity": "气道炎症",
"start_offset": 59,
"end_offset": 63,
"label": "dis"
},
{
"id": 5,
"entity": "糖皮质激素",
"start_offset": 65,
"end_offset": 70,
"label": "dru"
}
] |
常用药物为孟鲁司特钠（montelukastsodium），商品名为顺尔宁颗粒剂或咀嚼片：6～14岁5mg，2～5岁4mg，每晚服。 | [
{
"id": 0,
"entity": "孟鲁司特钠",
"start_offset": 5,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "montelukastsodium",
"start_offset": 11,
"end_offset": 28,
"label": "dru"
},
{
"id": 2,
"entity": "顺尔宁颗粒剂或咀嚼片",
"start_offset": 34,
"end_offset": 44,
"label": "dru"
}
] |
（五）肥大细胞膜稳定剂是一种非糖皮质激素类抗炎制剂，可抑制肥大细胞释放介质，对其他炎症细胞释放介质也有选择性抑制作用；主要用于轻中度哮喘患者。 | [
{
"id": 0,
"entity": "肥大细胞膜稳定剂",
"start_offset": 3,
"end_offset": 11,
"label": "dru"
},
{
"id": 1,
"entity": "非糖皮质激素类抗炎制剂",
"start_offset": 14,
"end_offset": 25,
"label": "dru"
},
{
"id": 2,
"entity": "肥大细胞",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "介质",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 4,
"entity": "炎症细胞",
"start_offset": 41,
"end_offset": 45,
"label": "bod"
},
{
"id": 5,
"entity": "介质",
"start_offset": 47,
"end_offset": 49,
"label": "bod"
},
{
"id": 6,
"entity": "轻中度哮喘",
"start_offset": 63,
"end_offset": 68,
"label": "dis"
}
] |
此类药物包括：色甘酸钠、尼多酸钠和酮替酚。 | [
{
"id": 0,
"entity": "色甘酸钠",
"start_offset": 7,
"end_offset": 11,
"label": "dru"
},
{
"id": 1,
"entity": "尼多酸钠",
"start_offset": 12,
"end_offset": 16,
"label": "dru"
},
{
"id": 2,
"entity": "酮替酚",
"start_offset": 17,
"end_offset": 20,
"label": "dru"
}
] |
（六）长效或缓释支气管扩张剂主要用于缓解期的轻中度咳喘症状，特别是夜间咳喘以及运动后咳喘。 | [
{
"id": 0,
"entity": "长效或缓释支气管扩张剂",
"start_offset": 3,
"end_offset": 14,
"label": "dru"
},
{
"id": 1,
"entity": "轻中度咳喘",
"start_offset": 22,
"end_offset": 27,
"label": "dis"
},
{
"id": 2,
"entity": "夜间咳喘",
"start_offset": 33,
"end_offset": 37,
"label": "dis"
},
{
"id": 3,
"entity": "运动后咳喘",
"start_offset": 39,
"end_offset": 44,
"label": "dis"
}
] |
1.长效或控释β2</sub>受体激动剂（1）沙丁胺醇缓释片（Etinoline）每片4mg。 | [
{
"id": 0,
"entity": "沙丁胺醇缓释片",
"start_offset": 23,
"end_offset": 30,
"label": "dru"
},
{
"id": 1,
"entity": "Etinoline",
"start_offset": 31,
"end_offset": 40,
"label": "dru"
}
] |
（2）丙卡特罗（Meptin）每片25μg。 | [
{
"id": 0,
"entity": "丙卡特罗",
"start_offset": 3,
"end_offset": 7,
"label": "dru"
},
{
"id": 1,
"entity": "Meptin",
"start_offset": 8,
"end_offset": 14,
"label": "dru"
}
] |
2.氨茶碱控释片（1）舒弗美：每片100mg。 | [
{
"id": 0,
"entity": "氨茶碱控释片",
"start_offset": 2,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "舒弗美",
"start_offset": 11,
"end_offset": 14,
"label": "dru"
}
] |
第二节偏头痛偏头痛（migraine）是一种反复发作的神经血管性头痛，多在单侧，每次发作性质与过程相似，间歇期正常。 | [
{
"id": 0,
"entity": "偏头痛",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "偏头痛",
"start_offset": 6,
"end_offset": 9,
"label": "dis"
},
{
"id": 2,
"entity": "神经血管性头痛",
"start_offset": 27,
"end_offset": 34,
"label": "dis"
}
] |
可伴发恶心、呕吐、视觉改变以及对光和声音的过度敏感等症状。 | [
{
"id": 0,
"entity": "恶心",
"start_offset": 3,
"end_offset": 5,
"label": "sym"
},
{
"id": 1,
"entity": "呕吐",
"start_offset": 6,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "视觉改变",
"start_offset": 9,
"end_offset": 13,
"label": "sym"
},
{
"id": 3,
"entity": "对光和声音的过度敏感",
"start_offset": 15,
"end_offset": 25,
"label": "sym"
}
] |
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